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  4. NBEAL2 Antibody, FITC conjugated

NBEAL2 Antibody, FITC conjugated

  • 中文名稱：
    NBEAL2兔多克隆抗體, FITC偶聯
  • 貨號：
    CSB-PA751159LC01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) NBEAL2 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    NBEAL2
  • 別名：
    BDPLT4 antibody; GPS antibody; NBEAL2 antibody; NBEL2_HUMAN antibody; Neurobeachin-like protein 2 antibody; UNQ253/PRO290 antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Neurobeachin-like protein 2 protein (1360-1574AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    FITC
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 產品提供形式：
    Liquid
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 功能：
    Probably involved in thrombopoiesis. Plays a role in the development or secretion of alpha-granules, that contain several growth factors important for platelet biogenesis.
  • 基因功能參考文獻：
    1. Author observed absence of NBEAL2 in platelets from GPS patients with 3 different genotypes, and reduced/truncated platelet NBEAL2 has been reported for others. PMID: 29869935
    2. NBEAL2 has an essential role in neutrophil and NK cell function and pathogen defense PMID: 28783043
    3. we herein show a long-distance regulatory region with GATA1 binding sites as being a strong enhancer for NBEAL2 expression. PMID: 28082341
    4. NBEAL2 mutation is associated with gray platelet syndrome. PMID: 21765411
    5. Mutations in NBEAL2, encoding a BEACH protein is associated with gray platelet syndrome. PMID: 21765413

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  • 相關疾病：
    Gray platelet syndrome (GPS)
  • 亞細胞定位：
    Endoplasmic reticulum.
  • 蛋白家族：
    WD repeat neurobeachin family
  • 組織特異性：
    Expressed in megakaryocytes.
  • 數據庫鏈接：

    HGNC: 31928

    OMIM: 139090

    KEGG: hsa:23218

    STRING: 9606.ENSP00000415034

    UniGene: Hs.437043



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